Abstract
The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.
Highlights
The exstrophy-epispadias complex (EEC) is a rare spectrum of defects affecting the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor musculature, and bony pelvis
These results led to the recent finding that insertion and deletion polymorphisms of ΔNp63 lead to the reduced p63 expression that may cause EEC [13]
When it is possible to place the bladder into a deep orthotopic position during complete primary repair of bladder exstrophy” (CPRE), the tubularized urethra is usually shorter than the actual corpora, resulting in hypospadias and necessitating further surgical repair and the attendant risk of further associated complications [51]
Summary
The exstrophy-epispadias complex (EEC) is a rare spectrum of defects affecting the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor musculature, and bony pelvis. The three most common presentations of EEC are epispadias, classic bladder exstrophy (CBE), and cloacal exstrophy (CE) (Figure 1). Complete epispadias is the least severe form of EEC and presents with a dorsally open urethral meatus with mild pubic diastasis and a closed anterior abdominal wall and bladder. CBE, the most common presentation of EEC, presents with a wide pubic diastasis and an abdominal wall defect exposing an open bladder and urethra with an epispadiac opening. Most patients with EEC undergo multiple reconstructive surgeries beginning with closure of the bony pelvis, bladder, and anterior abdominal wall, followed later by epispadias repair. While current techniques achieve reasonable success in preservation of renal function, continence, and cosmesis, there are many recognized complications associated with reconstruction
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